39 Days Smarter!!

1. 39 Days Smarter!! Morning report Tuesday, August 9th, 2011

2. CXR: ~Superior segment RLL PNA ~Early LLL

3. Complications of Sickle Cell Disease

4. Quick Note on Pathogenesis of SCD… Single nucleotide substitution in the beta globin gene on chromosome 11 substitution of valine for glutamic acid polymerization of HgS on deoxygenation multiple polymers bundle into rod-like structures that distort RBC into crescent shape vascular occlusion, organ ischemia, end organ damage AR in inheritance

5. Clinical Manifestations • Appear during first postnatal year • Due to decrease in HbF • Inhibits deoxy-HbS polymerization in RBC • Severity based on Hbvarient: • SS (Hb 6-8) • S-beta0-thalassemia (Hb 7-9) • SC (Hb 10-14) • S-beta+-thalassemia (Hb 9-12)

6. Clinical Complications of SCD • Young children • Bacterial infection • Splenic sequestration • Stroke • Adolescents and adults • Pulmonary HTN • Renal disease • Stroke • Avascular necrosis • Leg ulcers • Chronic pain syndromes

7. 2nd leading cause of admissions after VOC More common in children but more severe in adults Acute Chest Syndrome

8. Acute Chest Syndrome • Definition: • The radiologic appearance of new pulmonary infiltrate involving at least one complete lung segment plus one of the following • Fever >38.5 • Hypoxia • Chest pain • Signs of respiratory distress (tachypnea, wheezing, cough, retractions)

9. Acute Chest Syndrome • Triggers: • Infection (bacteria, viruses, Mycoplasma, Chlamydia) • VOC (fat embolism, hypoventilation) • Asthma • Treatment: • Broad spectrum Abx (cephalosporin + macrolide) • Oxygen (Pox>92%) • Hydration • Incentive Spirometry • Early intervention with simple transfusion • Exchange transfusion • Reserved for patients with progressive, multilobe infiltrates and hypoxia

10. Aplastic Crisis • Most common cause of transient red cell aplasia • Presentation: • Fever • URI Sx • Fatigue • Pallor (with absence of scleralicterus) • Decrease in baseline Hb with reticulocytopenia • 7-10 days • Dx: Parvovirus B19 IgM • Tx: Supportive care (transfusion)

11. Osteomyelitis • Salmonella*, S. aureus • Clinical presentation with significant overlap with VOC • No definitive imaging modality can differentiate with certainty • Diagnosis on clinical assessment • Fever • Leukocytosis • ESR • +BCx

12. Bacteremia/ Sepsis • Functional asplenia • At 1 yo 30% • At 6 yo 90% • Risk of S. pneumoniaebacteremia 400-fold higher in SCD • Prevention • PCN • Pneumococcal immunizations • ParenteralAbx (ceftriaxone) still standard of care with temp>101

13. Acute Splenic Sequestration • Occurs in children<3 yo with HbSS and at any age with the other HbSvarients • Clinical Characteristics • Sudden enlargement of spleen • 2 g/dL drop in HgBfrom baseline • Reticulocytosis • +/- Thrombocytopenia • Frequently results in circulatory collapse!!

14. Cerebrovascular Disease • Significant cause of morbidity in children with SCD • Stroke in HbSS patients • 11% overt stroke by age 20 • 75% ischemic or thrombotic • Additional 22% with silent strokes • Neuropsychometricdefecits learning and cognitive problems • Peak incidence 2-10 yo • Large artery disease • Internal carotid • Anterior and middle cerebral arteries

15. Cerebrovascular Disease • Prevention • TCD • Screening recommended in all children between 2-16y • Detecting children at risk • Flow velocity >200cm/sec • Chronic transfusion therapy • Goal: HbS <30% • Prevents second stroke in 80% • Reduces stroke risk 10 fold for patients with at risk TCDs

16. Chronic Organ Damage Pulmonary HTN Renal disease Avascular necrosis Leg ulcers Priapism Cholelithiasis Chronic pain syndromes

17. Thanks for your attention! Noon conference: cardiac surgical repair, Dr. Jason Turner